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DC Field | Value | Language |
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dc.contributor.author | Vassallo, Neville | - |
dc.contributor.author | Herms, Jochen | - |
dc.contributor.author | Kretzschmar, Hans A. | - |
dc.date.accessioned | 2014-12-04T14:18:09Z | - |
dc.date.available | 2014-12-04T14:18:09Z | - |
dc.date.issued | 2004 | - |
dc.identifier.citation | Malta Medical Journal. 2004, Vol.16(4), p. 15-19 | en_GB |
dc.identifier.uri | https://www.um.edu.mt/library/oar//handle/123456789/557 | - |
dc.description.abstract | Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of invariably fatal neurodegenerative disorders. Uniquely, they may present as sporadic, inherited, or infectious forms, all of which involve conversion of the normal cellular prion protein (PrPC) into a pathogenic likeness of itself (PrPSc). Formation of neurotoxic PrPSc and/or loss of the normal function of native PrPC result in activation of cellular pathways ultimately leading to neuronal death. Prion diseases can affect both humans and animals, with scrapie of sheep, bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease being the most notable. This review is intended to provide an overview of the salient scientific discoveries in prion research, mainly from a molecular perspective. Further, some of the major outstanding questions in prion science are highlighted. Prion research is having a profound impact on modern medicine, and strategies for prevention and treatment of these disorders may also find application in the more common neurodegenerative diseases. | en_GB |
dc.language.iso | en | en_GB |
dc.publisher | Malta Medical Journal | en_GB |
dc.rights | info:eu-repo/semantics/openAccess | en_GB |
dc.subject | Prion diseases -- Pathogenesis | en_GB |
dc.subject | Prions -- Research | en_GB |
dc.subject | Creutzfeldt-Jakob syndrome | en_GB |
dc.subject | Prion diseases -- Treatment | en_GB |
dc.title | The Molecular Pathology of Prion Diseases | en_GB |
dc.type | article | en_GB |
dc.rights.holder | The copyright of this work belongs to the author(s)/publisher. The rights of this work are as defined by the appropriate Copyright Legislation or as modified by any successive legislation. Users may access this work and can make use of the information contained in accordance with the Copyright Legislation provided that the author must be properly acknowledged. Further distribution or reproduction in any format is prohibited without the prior permission of the copyright holder. | en_GB |
dc.description.reviewed | peer-reviewed | - |
Appears in Collections: | MMJ, Volume 16, Issue 4 MMJ, Volume 16, Issue 4 Scholarly Works - FacM&SPB |
Files in This Item:
File | Description | Size | Format | |
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2004.Vol16.Issue4.A2.pdf | The Molecular Pathology of Prion Diseases | 87.05 kB | Adobe PDF | View/Open |
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